Making the diagnosis in hematology part 2

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Making the diagnosis in hematology part 2

  1. 1. Making the diagnosis in Hematology Part 2
  2. 2. Myeloproliferative disorders <ul><li>Polycythaemia </li></ul><ul><li>Essential thrombocythaemia </li></ul><ul><li>Myelofibrosis </li></ul><ul><li>Myelodysplasia. </li></ul>
  3. 3. Polycythaemia <ul><li>Absolute erythrocytosis. </li></ul><ul><li>Primary </li></ul><ul><li>Secondary </li></ul><ul><li>Relative polycythaemia (Gaisbock’s syndrome) </li></ul>
  4. 4. Causes of secondary Polycythaemia
  5. 5. Diagnosis of PV
  6. 6. Features suggestive of Polycythaemia? <ul><li>Hypertension </li></ul><ul><li>Angina </li></ul><ul><li>Intermittent claudication </li></ul><ul><li>Tendency to bleed </li></ul><ul><li>Severe itching after a hot bath . </li></ul><ul><li>Gout </li></ul><ul><li>Peptic ulceration. </li></ul>
  7. 7. Lab features to distinguish between primary and secondary polycythaemia <ul><li>In Primary , low serum EPO . </li></ul><ul><li>In secondary, EPO levels normal or raised. </li></ul>
  8. 8. Features of relative polycythaemia (Gaisbock's syndrome) <ul><li>An apparent rise of the erythrocyte level in the blood. </li></ul><ul><li>Normal red cell volume . </li></ul><ul><li>Reduced plasma volume . </li></ul><ul><li>Often caused by loss of body fluids, such as through burns, dehydration and stress . </li></ul>
  9. 9. Common scenario for relative polycythaemia <ul><li>Middle-aged men </li></ul><ul><li>Smokers </li></ul><ul><li>Obese </li></ul><ul><li>Hypertensive. </li></ul><ul><li>May present with cardiovascular problems such as myocardial or cerebral ischaemia. </li></ul>
  10. 10. Essential thrombocythaemia <ul><li>Patients have normal Hb levels and WBC but elevated platelet counts. </li></ul><ul><li>At diagnosis the platelet count will usually be 600 × 10 9 /L, and may be as high as 2000. </li></ul><ul><li>As a general rule, if a person who is clinically normal with good health has a very high platelet count (> 1000 × 109/L) will most likely prove to have ET. </li></ul><ul><li>JAK2 mutation in 50% of patients. </li></ul>
  11. 11. Myelofibrosis <ul><li>Blood film: Poikilocytes and red cells with characteristic tear-drop forms are seen. </li></ul><ul><li>The WBC >100. </li></ul><ul><li>High platelet count may be very high, but in later stages, thrombocytopenia occurs. </li></ul><ul><li>Bone marrow aspiration often unsuccessful. </li></ul><ul><li>A bone marrow trephine is necessary to show the markedly increased fibrosis . </li></ul>
  12. 12. <ul><li>Increased numbers of megakaryocytes may be seen. </li></ul><ul><li>The Philadelphia chromosome is absent. </li></ul><ul><li>The leucocyte alkaline phosphatase (LAP) score is normal or high . </li></ul><ul><li>High serum urate is present. </li></ul><ul><li>Low serum folate levels </li></ul>
  13. 13. Lab investigation to help distinguish between myelofibrosis vs CML <ul><li>Appearance of the bone marrow and the absence of the Philadelphia chromosome in myelofibrosis. </li></ul>
  14. 14. Myelodysplasia <ul><li>Pancytopenia: </li></ul><ul><li>anaemia </li></ul><ul><li>neutropenia </li></ul><ul><li>monocytosis </li></ul><ul><li>Thrombocytopenia </li></ul><ul><li>bone marrow usually shows increased cellularity despite the pancytopenia. </li></ul><ul><li>Ring sideroblasts are present in all types. </li></ul>

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